Wellbeing in Children and Adolescents with Fontan Physiology.

OBJECTIVE: To assess health-related quality of life (HRQOL) and global quality of life (QOL) in children and adolescents with Fontan physiology and identify key predictors influencing these outcomes. STUDY DESIGN: Cross-sectional analysis of 73 children and adolescents enrolled in the Australia and New Zealand Fontan Registry (ANZFR) aged 6-17 years, at least 12 months post-Fontan operation. Assessments included the Pediatric Quality of Life Inventory 4.0 (PedsQL) for HRQOL and a developmentally-tailored visual analogue scale (0-10) for global QOL, along with validated sociodemographic, clinical, psychological, relational, and parental measures. Clinical data were provided by the ANZFR. RESULTS: Participants (mean age: 11.5±2.6 years, 62% male) reported lower overall HRQOL (p<0.001), and lower scores across all HRQOL domains (all p<0.0001), compared with normative data. Median global QOL score was 7.0 (IQR 2.2), with most participants (79%) rating their global QOL ≥6. Anxiety and depressive symptoms requiring clinical assessment were reported by 21% and 26% of participants, respectively. Age, sex, and perceived seriousness of CHD explained 15% of the variation in HRQOL scores, while depressive symptoms and treatment-related anxiety explained an additional 37% (final model: 52% of variance explained). For global QOL, sociodemographic and clinical factors explained 13% of the variance in scores, while depressive symptoms explained a further 25% (final model: 38% of variance explained). Parental factors were not associated with child QOL outcomes. CONCLUSIONS: Children and adolescents with Fontan physiology experience lower HRQOL than community-based norms, despite reporting fair overall QOL. Psychological factors predominantly influenced QOL outcomes, indicating strategies to bolster psychological health could improve QOL in this population.

Shear-wave elastography for monitoring Fontan-associated liver disease: A prospective cohort study.

The spectrum of Fontan-associated liver disease (FALD) varies from abnormal liver function tests to fibrosis and even cirrhosis. In this prospective study, we evaluated the role of shear-wave elastography (SWE) in predicting the presence of advanced FALD. Forty-eight patients (30 males, 13.9 [6-21] years) with a Fontan circulation were evaluated at 8.3 (2.1-18.7) years since the Fontan surgery. The median liver stiffness measurement (LSM) value was higher than values in normal children at 15.4 (9.5-38.7) kPa. The LSMs had a weak but significant correlation with age at the time of LSM (r = 0.25, p = 0.01) and duration post-Fontan surgery (r = 0.31, p = 0.02). It had a poor correlation with the concomitant aspartate transaminase-to-platelet ratio index (r = 0.1, p = 0.39). No difference in the elastography values between children with and without ultrasound evidence of advanced liver disease (17.7 [interquartile range, IQR: 4] vs. 16.1 [IQR: 6], p = 0.62] was observed. Further studies are required to determine the precise role of SWE as a noninvasive marker of liver fibrosis in FALD.

Implementing neurodevelopmental follow-up care for children with congenital heart disease: A scoping review with evidence mapping.

AIM: To identify and map evidence describing components of neurodevelopmental follow-up care for children with congenital heart disease (CHD). METHOD: This was a scoping review of studies reporting components of neurodevelopmental follow-up programmes/pathways for children with CHD. Eligible publications were identified through database searches, citation tracking, and expert recommendations. Two independent reviewers screened studies and extracted data. An evidence matrix was developed to visualize common characteristics of care pathways. Qualitative content analysis identified implementation barriers and enablers. RESULTS: The review included 33 studies. Twenty-one described individual care pathways across the USA (n = 14), Canada (n = 4), Australia (n = 2), and France (n = 1). The remainder reported surveys of clinical practice across multiple geographical regions. While heterogeneity in care existed across studies, common attributes included enrolment of children at high-risk of neurodevelopmental delay; centralized clinics in children's hospitals; referral before discharge; periodic follow-up at fixed ages; standardized developmental assessment; and involvement of multidisciplinary teams. Implementation barriers included service cost/resourcing, patient burden, and lack of knowledge/awareness. Multi-level stakeholder engagement and integration with other services were key drivers of success. INTERPRETATION: Defining components of effective neurodevelopmental follow-up programmes and care pathways, along with enhancing and expanding guideline-based care across regions and into new contexts, should continue to be priorities. WHAT THIS PAPER ADDS: Twenty-two different neurodevelopmental follow-up care pathways/programmes were published, originating from four countries. Twelve additional publications described broad practices for neurodevelopmental follow-up across regions Common attributes across eligibility, service structure, assessment processes, and care providers were noted. Studies reported programme acceptability, uptake, cost, and effectiveness. Implementation barriers included service cost/resourcing, patient burden, and lack of knowledge/awareness.

Closing the gap between acceptable and ideal in catheterisation for paediatric and congenital heart disease-A global view.

In recent issues of the Journal of the Society for Cardiovascular Angiography and Interventions and the Journal of the American College of Cardiology: Cardiovascular Interventions, Holzer and colleagues presented an Expert Consensus Document titled: "PICS / AEPC / APPCS / CSANZ / SCAI / SOLACI: Expert consensus statement on cardiac catheterization for pediatric patients and adults with congenital heart disease." This Expert Consensus Document is a massively important contribution to the community of paediatric and congenital cardiac care. This document was developed as an Expert Consensus Document by the Pediatric and Congenital Interventional Cardiovascular Society, the Association for European Paediatric and Congenital Cardiology, the Asia-Pacific Pediatric Cardiac Society, the Cardiac Society of Australia and New Zealand, the Society for Cardiovascular Angiography and Interventions, and the Latin American Society of Interventional Cardiology, as well as the Congenital Cardiac Anesthesia Society and the American Association of Physicists in Medicine.As perfectly stated in the Preamble of this Expert Consensus Document, "This expert consensus document is intended to inform practitioners, payors, hospital administrators and other parties as to the opinion of the aforementioned societies about best practices for cardiac catheterisation and transcatheter management of paediatric and adult patients with congenital heart disease, with added accommodations for resource-limited environments." And, the fact that the authorship of this Expert Consensus Document includes global representation is notable, commendable, and important.This Expert Consensus Document has the potential to fill an important gap for this patient population. National guideline documents for specific aspects of interventions in patients with paediatric heart disease, including training guidelines, do exist. However, this current Expert Consensus Document authored by Holzer and colleagues provides truly globally applicable standards on cardiac catheterisation for both paediatric patients and adults with congenital heart disease (CHD).Our current Editorial provides different regional perspectives from senior physicians dedicated to paediatric and congenital cardiac care who are practicing in Europe, the Asia-Pacific region, Latin America, Australia/New Zealand, and North America. Establishing worldwide standards for cardiac catheterisation laboratories for children and adults with CHD is a significant stride towards improving the quality and consistency of care. These standards should not only reflect the current state of medical knowledge but should also be adaptable to future advancements, ultimately fostering better outcomes and enhancing the lives of individuals affected by CHD worldwide.Ensuring that these standards are accessible and adaptable across different healthcare settings globally is a critical step. Given the variability in resources and infrastructure globally, the need exists for flexibility and tailoring to implement recommendations.The potential impact of the Expert Consensus Document and its recommendations is likely significant, but heterogeneity of healthcare systems will pose continuing challenges on healthcare professionals. Indeed, this heterogeneity of healthcare systems will challenge healthcare professionals to finally close the gap between acceptable and ideal in the catheterisation of patients with paediatric and/or congenital heart disease.

Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards). 1st Edition.

These first Australian National Standards of Care for Childhood-onset Heart Disease (CoHD Standards) have been developed to inform the healthcare requirements for CoHD services and enable all Australian patients, families and carers impacted by CoHD (paediatric CoHD and adult congenital heart disease [ACHD]) to live their best and healthiest lives. The CoHD Standards are designed to provide the clarity and certainty required for healthcare services to deliver excellent, comprehensive, inclusive, and equitable CoHD care across Australia for patients, families and carers, and offer an iterative roadmap to the future of these services. The CoHD Standards provide a framework for excellent CoHD care, encompassing key requirements and expectations for whole-of-life, holistic and connected healthcare service delivery. The CoHD Standards should be implemented in health services in conjunction with the National Safety and Quality Health Service Standards developed by the Australian Commission on Safety and Quality in Health Care. All healthcare services should comply with the CoHD Standards, as well as working to their organisation's or jurisdiction's agreed clinical governance framework, to guide the implementation of structures and processes that support safe care.

Systematic Review of Instruments Assessing Psychosocial Adaptation and Outcomes Among Families of Children With Congenital Heart Disease.

OBJECTIVE: This systematic review identified instruments quantitatively assessing psychosocial adaptation and outcomes in families of children with congenital heart disease (CHD) and evaluated instrument psychometrics. METHODS: Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and a prospectively registered protocol, electronic databases (CINAHL, Embase, PubMed/MEDLINE, PsycINFO, and SCOPUS) were searched from inception until June 20, 2021 for peer-reviewed articles published in English, reporting quantitative data on psychosocial outcomes among parents/caregivers, siblings, or family system. Instrument characteristics and psychometrics were extracted, and adapted COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) criteria were applied to assess instrument quality. Descriptive statistics and narrative synthesis were used for analysis. RESULTS: Overall, 108 articles reporting on 107 distinct samples across 26 countries met inclusion. Across those articles, 40 instruments assessed psychological functioning or distress, 12 assessed coping, 11 assessed quality of life constructs, 10 assessed parenting stress/caregiver burden, 10 assessed family functioning/impact, 10 assessed stress appraisal, 5 assessed sibling psychosocial outcomes, and 2 assessed couple relationship satisfaction/strain. Applying COSMIN criteria to available data on original instrument development articles/manuals for English language instruments (n = 54), 67% scored a positive property evidence rating for content validity, 39% for internal consistency, 4% for test-retest reliability, and 9% for responsiveness (longitudinal validity). CONCLUSIONS: Studies vary widely in instruments used to assess psychosocial adaptation and outcomes among families of children with CHD. Instrument selection informed by robust key psychometrics, increased psychometric reporting, development of both a "toolkit" approach and a comprehensive CHD-specific family instrument are among key recommendations.

Clinical stakeholder preferences for paediatric cardiac surgery outcome reporting in Australia and New Zealand.

BACKGROUND: Outcome reporting is an essential element of quality assurance. Evaluation of the information needs of stakeholders of outcome reporting is limited. This study aimed to examine stakeholder preferences for the content, format, and dissemination of paediatric cardiac surgery performance data in Australia and New Zealand. METHODS: Semi-structured interviews were completed with a purposive sample of Queensland stakeholders to evaluate their attitudes and expectations regarding reporting of paediatric cardiac surgery outcomes. The interviews were audio-recorded and transcribed. Two researchers used an interpretive description approach to analyse the transcripts qualitatively. RESULTS: Nineteen stakeholders were interviewed including fifteen clinicians, four parents, one hospital administrator, and one consumer advocate were interviewed. Mortality was highlighted as the area of greatest interest in reports by clinical and consumer groups. The majority preferred hospital rather than individual/clinician-level reporting. Annual reports were preferred by clinicians who requested reports be distributed electronically. CONCLUSIONS: The evidence generated from outcome reporting in paediatric cardiac surgery is highly desired by clinicians, administrators, parents, families, and advocacy groups. Clinical users prefer information to assist in clinical decision-making, while families seek personalised information at crucial time points in their clinical journey.

Early Experience With the Bentley BeGraft Aortic Stent for the Management of Aortic Arch Pathology in the Paediatric Population.

BACKGROUND: Aortic arch pathology in older children is often treated preferentially with stenting. Both bare metal and covered stents have been utilised, with potential advantages of covered stents. The search for the ideal covered stent continues. METHODS: Retrospective review of all paediatric patients undergoing treatment of aortic arch pathology utilising the Bentley BeGraft Aortic stent (BeGraft Aortic, Bentley InnoMed, Hechingen, Germany) from June 2017 to May 2021. Outcome measures were procedural success, complications, medium-term patency and need for re-intervention. RESULTS: Fourteen (14) stents were placed in 12 children (seven males). Indications were coarctation of the aorta in 10 and aneurysm in two. Median age was 11.8 years (8.7-16.6 years) and median weight 42.5 kg (24.8-84 kg). Median coarctation narrowing of 4 mm (range 1-9 mm), improved to 11 mm (range 9-15 mm). The median coarctation gradient improved from 32 mmHg (range 11-42 mmHg) to 7 mmHg (range 0-14 mmHg). Both aneurysms were successfully occluded. There was no mortality or major morbidity. In one patient balloon rupture occurred requiring a second balloon for full inflation and one patient had a minor access site bleed. Follow-up median was 28 months (range 13-65 months). One patient underwent repeat balloon dilation for increased blood pressure gradient at 47 months post implant and a second patient additional stent insertion for a mid-stent aneurysm at 65 months. CONCLUSION: The Bentley BeGraft Aortic stent can be safely deployed in children for the treatment of aortic arch pathology. Medium-term patency is acceptable. Longer term follow-up in larger series will be required to assess stent performance.

Integrated guidance to enhance the care of children and adolescents with familial hypercholesterolaemia: Practical advice for the community clinician.

Familial hypercholesterolaemia (FH) is a highly penetrant monogenic disorder present from birth that markedly elevates plasma low-density lipoprotein (LDL)-cholesterol (LDL-C) concentration and, if untreated, leads to premature atherosclerosis and coronary artery disease (CAD). At a prevalence of 1:250 individuals, with over 90% undiagnosed, recent estimates suggest that there are approximately 22 000 children and adolescents with FH in Australia and New Zealand. However, the overwhelming majority remain undetected and inadequately treated until adulthood or after their first cardiac event. The guidance in this paper aims to increase awareness about paediatric FH and provide practical advice for the diagnosis and management of FH in children and adolescents. Recommendations are given on the detection, diagnosis, assessment and management of FH in children and adolescents. Recommendations are also made on genetic testing, including counselling and the potential for universal screening programmes. Practical guidance on management includes treatment of non-cholesterol risk factors, and safe and appropriate use of LDL-C lowering therapies, including statins, ezetimibe, PCSK9 inhibitors and lipoprotein apheresis. Models of care for FH need to be adapted to local and regional health care needs and available resources. Targeting the detection of FH as a priority in children and young adults has the potential to alter the natural history of atherosclerotic cardiovascular disease and recognise the promise of early detection for improving long-term health outcomes. A comprehensive implementation strategy, informed by further research, including assessments of cost-benefit, will be required to ensure that this new guidance benefits all families with or at risk of FH.

Essentials of a new clinical practice guidance on familial hypercholesterolaemia for physicians.

Familial hypercholesterolaemia (FH) is a common, heritable and preventable cause of premature coronary artery disease. New clinical practice recommendations are presented to assist practitioners in enhancing the care of all patients with FH. Core recommendations are made on the detection, diagnosis, assessment and management of adults, children and adolescents with FH. Management is under-pinned by the precepts of risk stratification, adherence to healthy lifestyles, treatment of non-cholesterol risk factors and appropriate use of low-density lipoprotein (LDL)-cholesterol-lowering therapies including statins, ezetimibe and proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors. The recommendations need to be utilised using judicious clinical judgement and shared decision-making with patients and families. New government-funded schemes for genetic testing and use of PCSK9 inhibitors, as well as the National Health Genomics Policy Framework, will enable adoption of the recommendations. However, a comprehensive implementation science and practice strategy is required to ensure that the guidance translates into benefit for all families with FH.

Balloon dilatation versus surgical valvotomy for congenital aortic stenosis: a propensity score matched study.

BACKGROUND: Balloon valvuloplasty and surgical aortic valvotomy have been the treatment mainstays for congenital aortic stenosis in children. Choice of intervention often differs depending upon centre bias with limited relevant, comparative literature. OBJECTIVES: This study aims to provide an unbiased, contemporary matched comparison of these balloon and surgical approaches. METHODS: Retrospective analysis of patients with congenital aortic valve stenosis who underwent balloon valvuloplasty (Queensland Children's Hospital, Brisbane) or surgical valvotomy (Royal Children's Hospital, Melbourne) between 2005 and 2016. Patients were excluded if pre-intervention assessment indicated ineligibility to either group. Propensity score matching was performed based on age, weight, and valve morphology. RESULTS: Sixty-five balloon patients and seventy-seven surgical patients were included. Overall, the groups were well matched with 18 neonates/25 infants in the balloon group and 17 neonates/28 infants in the surgical group. Median age at balloon was 92 days (range 2 days - 18.8 years) compared to 167 days (range 0 days - 18.1 years) for surgery (rank-sum p = 0.08). Mean follow-up was 5.3 years. There was one late balloon death and two early surgical deaths due to left ventricular failure. There was no significant difference in freedom from reintervention at latest follow-up (69% in the balloon group and 70% in the surgical group, p = 1.0). CONCLUSIONS: Contemporary analysis of balloon aortic valvuloplasty and surgical aortic valvotomy shows no difference in overall reintervention rates in the medium term. Balloon valvuloplasty performs well across all age groups, achieving delay or avoidance of surgical intervention.

The Gore Septal Occluder (GSO) for Multiple Indications in Children - An Intention to Treat Analysis.

OBJECTIVE: To assess the safety and efficacy of all uses of the Gore septal occluder (GSO) (WL Gore and Associates, Flagstaff, AZ, USA) in a tertiary children's hospital. BACKGROUND: The GSO is widely used in atrial septal communication closure in the adult population. Usage in the paediatric population is expanding. Device design potentially confers some benefits in this population. METHODS: Single centre retrospective data review over 6 years to July 2019 in a tertiary paediatric cardiac centre. All instances of intended GSO use were identified. Outcome measures were closure rates and procedural complications. RESULTS: Fifty-four (54) patients with median age 7.9 years (3-17 years) and weight 26.5 kg (12.6-76 kg) underwent attempted GSO implantation. Indications were atrial septal defect (ASD) with volume loading (39); abolition of right to left shunt (8); stroke prevention (3) and Fontan fenestration (4). Successful GSO deployment in 46/50 (92%) of the ASD/PFO group and 100% of the Fontan group. Occlusion rate was assessed at 24 hours and at medians of 1.4 months (IQR 1.1 mo), 9 months (IQR 8.3 mo) and 25.4 months (IQR 28.1 mo). Complete occlusion documented at these points was 91%, 87%, 93% and 98% respectively. Major complications prevented GSO implantation in four patients. Two (2) patients had transient rhythm disorder. There was no incidence of erosion, further arrhythmia, venous access complications or death during follow-up. CONCLUSIONS: The GSO can be safely deployed in both the atrial septum and Fontan fenestration with a low rate of complications. Trivial residual shunt is present in a number of cases early after deployment, although improves with time. Delivery sheath size was not a cause of significant complications in this patient cohort.

Prevalence and Cost of Managing Paediatric Cardiac Disease in Queensland.

BACKGROUND: There is a paucity of contemporary information regarding the prevalence and related health care expenditure of the most common cardiac conditions in Australian children, including congenital heart disease (CHD). METHODS: The 10 most common cardiac conditions managed by Queensland Paediatric Cardiac Service during 2014-2015 were identified from an electronic database: ventricular septal defect (VSD), pulmonary stenosis, aortic stenosis, tetralogy of Fallot, atrioventricular septal defect, transposition, Ebstein's anomaly, long QT syndrome, dilated cardiomyopathy, and rheumatic carditis. Demographic data, clinic attendance, investigational procedures, and therapeutic interventions were extracted from the electronic health records to derive indicative population estimates and direct health care expenditure relating to CHD. RESULTS: A total of 2,519 patients diagnosed with the 10 target conditions were being actively managed, including 456 (18.1%) new-born and 787 prevalent cases (2.5/1,000 population) aged <5 years. A total of 12,180 (4.8/case) investigations were performed (6,169 echocardiographic and 279 cardiac catheterisation procedures) costing $2.25 million/annum. A further 5,326 clinic visits (2.1/case, 22% regional) were conducted at a cost of $550,000/annum. A combined total of 804 catheter-based interventions and surgical procedures were performed in 300 cases (11.9%) at a cost of $13.6 million/annum. VSD (38.6% of cases) was the single greatest contributor ($5.1 million/annum) to total combined direct health care costs of $13.6 million/annum for the 2,519 patients. CONCLUSIONS: These pilot data indicate a significant patient population and health care burden imposed by CHD in Queensland. Future initiatives to better quantify this burden, from an individual to health system perspective, are urgently needed.

Integrated Guidance for Enhancing the Care of Familial Hypercholesterolaemia in Australia.

Familial hypercholesterolaemia (FH) is a dominant and highly penetrant monogenic disorder present from birth that markedly elevates plasma low-density lipoprotein (LDL)-cholesterol concentration and, if untreated, leads to premature atherosclerosis and coronary artery disease (CAD). There are approximately 100,000 people with FH in Australia. However, an overwhelming majority of those affected remain undetected and inadequately treated, consistent with FH being a leading challenge for public health genomics. To further address the unmet need, we provide an updated guidance, presented as a series of systematically collated recommendations, on the care of patients and families with FH. These recommendations have been informed by an exponential growth in published works and new evidence over the last 5 years and are compatible with a contemporary global call to action on FH. Recommendations are given on the detection, diagnosis, assessment and management of FH in adults and children. Recommendations are also made on genetic testing and risk notification of biological relatives who should undergo cascade testing for FH. Guidance on management is based on the concepts of risk re-stratification, adherence to heart healthy lifestyles, treatment of non-cholesterol risk factors, and safe and appropriate use of LDL-cholesterol lowering therapies, including statins, ezetimibe, proprotein convertase subtilisin/kexin type 9 inhibitors and lipoprotein apheresis. Broad recommendations are also provided for the organisation and development of health care services. Recommendations on best practice need to be underpinned by good clinical judgment and shared decision making with patients and families. Models of care for FH need to be adapted to local and regional health care needs and available resources. A comprehensive and realistic implementation strategy, informed by further research, including assessments of cost-benefit, will be required to ensure that this new guidance benefits all Australian families with or at risk of FH.

Neurodevelopmental and health-related quality-of-life outcomes in adolescence after surgery for congenital heart disease in infancy.

AIM: To assess outcomes in adolescence after surgery for congenital heart disease (CHD) in infancy. Domains analysed included cognition and executive function, social and emotional well-being, adaptive behaviour, academic achievement, and health-related quality of life (HRQoL). METHOD: Twenty-one participants (10 males, 11 females) ranged in age from 14 to 17 years (mean 15y 4.8mo, SD 8.4mo). Twenty had biventricular repairs. All were classified as New York Heart Association class I. Measures included: Wechsler Intelligence and Achievement scales; Wide Range Assessment of Memory and Learning, Second Edition; California Verbal Learning Test - Children's Version; Behaviour Rating Inventory of Executive Function; Conners, Third Edition; Adaptive Behavior Assessment System, Second Edition; Behavior Assessment System for Children, Second Edition; Rey-Osterrieth Complex Figure; and Pediatric Quality of Life Inventory. RESULTS: Outcomes were significantly lower (p≤0.01) than population norms for processing speed, mathematical achievement, attention, and visual-spatial ability. Participants reported more frequent learning problems but more positive family relations. HRQoL was significantly lower across most domains by self- and parent-proxy report. INTERPRETATION: Individuals with CHD may experience difficulties across a range of domains. These findings emphasize the importance of comprehensive screening, early intervention, and long-term follow-up, as deficits may extend into young adulthood. WHAT THIS PAPER ADDS: Identified cognitive, learning, and attentional impairments in adolescents after congenital heart disease surgery in infancy. Combined self-report, caregiver report, and laboratory tasks in a comprehensive neurodevelopmental assessment protocol. Health-related quality of life was lower across most domains.

Queensland Paediatric Cardiac Service: Lessons from evolution to a unified service in a tertiary children's hospital.

We describe a complex change process for the paediatric cardiac service in Queensland that involved transitioning the service out of an essentially adult hospital into one of two children's hospitals in Brisbane. This initial step was complex as the governance was changed from Queensland Health to Mater Health, an independent faith-based organisation who became the new employer. Six years later, the service was again transitioned; this time to the newly constructed Queensland Children's Hospital, with a Hospital and Health Services Board as the employer under the aegis of Queensland Health. This was a complex journey. As with all change processes there was resistance to change on the part of some individuals. Five years on from the second major change, the service is settled, has an excellent workplace culture, has excellent clinical outcomes and has become research intensive.

Congenital Heart Disease Long-term Improvement in Functional hEalth (CHD LIFE): A partnership programme to improve the long-term functional health of children with congenital heart disease in Queensland.

Children who undergo open-heart surgery in the first year of life for congenital heart disease (CHD) are at high-risk for impaired development across multiple domains. International recommendations include systematic periodic developmental surveillance into adolescence and the establishment of long-term follow-up programmes. This article describes the establishment and evolution of the Queensland Paediatric Cardiac Service neurodevelopmental follow-up programme - CHD LIFE (Long-term Improvement in Functional hEalth). Contextualising best practice recommendations to ensure a family-centred and sustainable approach to understand and support the long-term functional health needs of high-risk children with CHD as standard care was needed. We describe the transition from a centralised pilot Programme to the implementation of an integrated statewide approach aimed at delivering consistent high-level standards of care and a platform to evaluate therapeutic interventions.

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement.

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

Long-Term Outcomes of Hypertrophic Cardiomyopathy Diagnosed During Childhood: Results From a National Population-Based Study.

BACKGROUND: Late survival and symptomatic status of children with hypertrophic cardiomyopathy have not been well defined. We examined long-term outcomes for pediatric hypertrophic cardiomyopathy. METHODS: The National Australian Childhood Cardiomyopathy Study is a longitudinal population-based cohort study of children (0-10 years of age) diagnosed with cardiomyopathy between 1987 and 1996. The primary study end point was time to death or cardiac transplantation. RESULTS: There were 80 patients with hypertrophic cardiomyopathy, with a median age at diagnosis of 0.48 (interquartile range, 0.1, 2.5) years. Freedom from death/transplantation was 86% (95% confidence interval [CI], 77.0-92.0) 1 year after presentation, 80% (95% CI, 69.0-87.0) at 10 years, and 78% (95% CI, 67.0-86.0) at 20 years. From multivariable analyses, risk factors for death/transplantation included symmetrical left ventricular hypertrophy at the time of diagnosis (hazard ratio, 4.20; 95% CI, 1.60-11.05; P=0.004), Noonan syndrome (hazard ratio, 2.88; 95% CI, 1.02-8.08; P=0.045), higher posterior wall thickness z score (hazard ratio, 1.45; 95% CI, 1.22-1.73; P<0.001), and lower fractional shortening z score (hazard ratio, 0.84; 95% CI, 0.74-0.95; P=0.005) during follow-up. Nineteen (23%) subjects underwent left ventricular myectomy. At a median of 15.7 years of follow-up, 27 (42%) of 63 survivors were treated with ß-blocker, and 13 (21%) had an implantable cardioverter-defibrillator. CONCLUSIONS: The highest risk of death or transplantation for children with hypertrophic cardiomyopathy is within 1 year after diagnosis, with low attrition rates thereafter. Many subjects receive medical, surgical, or device therapy.

Long-Term Outcomes of Childhood Left Ventricular Noncompaction Cardiomyopathy: Results From a National Population-Based Study.

BACKGROUND: Long-term outcomes for childhood left ventricular noncompaction (LVNC) are uncertain. We examined late outcomes for children with LVNC enrolled in a national population-based study. METHODS: The National Australian Childhood Cardiomyopathy Study includes all children in Australia with primary cardiomyopathy diagnosed before 10 years of age between 1987 and 1996. Outcomes for subjects with LVNC with a dilated phenotype (LVNC-D) were compared with outcomes for those with dilated cardiomyopathy. Propensity-score analysis was used for risk factor adjustment. RESULTS: There were 29 subjects with LVNC (9.2% of all cardiomyopathy subjects), with a mean annual incidence of newly diagnosed cases of 0.11 per 100 000 at-risk individuals. Congestive heart failure was the initial symptom in 24 of 29 subjects (83%), and 27 (93%) had LVNC-D. The median age at diagnosis was 0.3 (interquartile interval, 0.08-1.3) years. The median duration of follow-up was 6.8 (interquartile interval, 0.7-24.0) years for all subjects and 24.7 (interquartile interval, 23.3 - 27.7) years for surviving subjects. Freedom from death or transplantation was 48% (95% confidence interval [CI], 30-65) at 10 years after diagnosis and 45% (95% CI, 27-63) at 15 years. In competing-risk analysis, 21% of subjects with LVNC were alive with normal left ventricular systolic function, and 31% were alive with abnormal function at 15 years. Propensity-score matching between subjects with LVNC-D and those with dilated cardiomyopathy suggested a lower freedom from death/transplantation at 15 years after diagnosis in the subjects with LVNC-D (LVNC-D, 46% [95% CI, 26-66] versus dilated cardiomyopathy, 70% [95% CI, 42-97]; P=0.08). Using propensity-score inverse probability of treatment-weighted Cox regression, we found evidence that LVNC-D was associated with a greater risk of death or transplantation (hazard ratio, 2.3; 95% CI, 1.4-3.8; P=0.0012). CONCLUSIONS: Symptomatic children with LVNC usually present in early infancy with a predominant dilated phenotype. Long-term outcomes are worse than for matched children with dilated cardiomyopathy.